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alpha-L-Iduronidase/IDUA Antibody [Biotin]

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Product Details

Summary
Applications WB
Clonality
Polyclonal
Host
Sheep
Conjugate
Biotin
Concentration
LYOPH

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alpha-L-Iduronidase/IDUA Antibody [Biotin] Summary

Immunogen
Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA
Ala26-Pro653 (Ala26Thr)
Accession # AAA81589
Specificity
Detects human alpha ‑L‑Iduronidase/IDUA in Western blots.
Source
N/A
Isotype
IgG
Clonality
Polyclonal
Host
Sheep
Gene
IDUA
Purity Statement
Antigen Affinity-purified
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Packaging, Storage & Formulations

Storage
Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
Buffer
Lyophilized from a 0.2 μm filtered solution in PBS with BSA as a carrier protein.
Preservative
No Preservative
Concentration
LYOPH
Reconstitution Instructions
Reconstitute at 0.2 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Background

alpha -L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non‑reducing terminal alpha -L-iduronic acid residues in GAGs including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits (2).

  1. Scott, H.S. et al. (1995) Hum. Mutat. 6:288.
  2. Wraith, J.E. (2005) Expert Opin. Pharmacother. 6:489.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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Bioinformatics

Gene Symbol IDUA